Vasculitis is a rare condition that occurs when your body’s immune system mistakenly attacks its own blood vessels. The job of the blood vessels is to carry blood from your heart to the rest of your organs.

Your body’s natural response when blood vessels are attacked is inflammation. Chronic inflammation of the vessels can lead to narrowing and disruption of blood flow to the rest of the body. This can cause serious complications, depending upon the severity of the disease, where it occurs in your body and the type of vasculitis.

Vasculitis can be life-threatening, but many treatment and management options exist. Vasculitis is a chronic disease. Once you have been diagnosed with vasculitis, the goal is to achieve remission (inactive disease), but relapses and flares of the disease can occur over time.

Behcet’s disease: This disease can cause inflammation in the arteries and veins. Signs and symptoms include oral and genital ulcers, eye inflammation and skin lesions.

Buerger's disease: This disease can cause inflammation and clots in the blood vessels that supply your hands and feet. It can cause pain in the extremities and ulcers on your fingers and toes. This disorder is associated with cigarette smoking.

Central nervous system vasculitis: This condition is inflammation of blood vessel walls in the brain or spine, which make up the central nervous system.

Cogan’s syndrome: This condition is systemic, meaning it affects the whole body, but is commonly seen in the aorta, which carries blood from the heart to the body.

Cryoglobulinemic Vasculitis: This disease can results from abnormal protein in the blood. It can be associated with infectious diseases such as Hepatitis C and also with underlying blood cancers. Signs and symptoms include rashes, joint pain, numbness, and weakness.

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss): This condition is associated with increased eosinophils (a type of white blood cell) and can affect the kidneys, lungs and nerves. It is often associated with asthma

Granulomatosis with Polyangiitis (Wegeners or GPA): This is another one of the ANCA-associated vasculitides. It can affect the sinuses, upper airway (trachea), lungs, kidneys and many other organs. Patients can experience joint pains, eye inflammation or redness, cough, blood in the urine, hearing or vision problems, rashes, as well as other symptoms of fatigue, weakness and fevers.

Henoch-Schonlein Purpura (HSP): This condition usually affects children, but can occur at any age. It results from inflammation in the capillaries (the smallest blood vessels) and can cause damage to the skin, joints, bowel and kidney. Signs and symptoms include abdominal pain, blood in the urine, joint pain, and a rash on your buttocks or lower legs.

Hypersensitivity vasculitis: This condition is often brought on by an allergic reaction to medication. It affects the skin, typically on the lower body.

Kawasaki disease: This disease affects young children and is associated with high abrupt fevers, redness in the eyes, rashes, swelling of the hands and feet, swollen glands in the neck, and redness/cracking of the tongue and lips. It has a good prognosis with treatment, but in some cases can lead to heart abnormalities.

Microscopic polyangiitis: This condition impacts the small blood vessels of the kidney and lungs and can have symptoms that come on suddenly and progress quickly.

Polyarteritis nodosa (PAN): This type of vasculitis affects the medium and small sized arteries. It can result in thinning of blood vessel wall that result in aneurysms (outpouchings of the blood vessels). Some of the symptoms include weight loss, abdominal pain, nerve and motor problems, testicle pain, and skin ulcerations or nodules

Polymyalgia rheumatica: This condition affects the body’s large joints and causes pain in areas like the lower back, shoulders, hips and thighs.

Takayasu's disease: This is a type of vasculitis affecting the large arteries in the body (the aorta and its branches).  It classically affects young women, and can be associated with high blood pressure in the body or in the blood vessel supplying the lungs. The disease can lead to narrowing and blockages or areas of weakness with dilation or aneurysms of the blood vessel. Symptoms include fatigue, weight loss, low grade fevers, arm or chest pain, decreased pulses, and possibly heart failure.

Temporal Arteritis(Giant cell arteritis): This condition usually affects older individuals (>50) and is associated with severe headaches. It can also cause scalp tenderness, jaw pain, changes in vision. If it is left untreated, it can lead to permanent vision changes and blindness. This disease is associated with polymyalgia rheumatic (PMR), which causes pain and soreness in the muscles of the shoulders/neck and hips.

The symptoms of vasculitis vary greatly depending upon the area affected by the condition. There are a handful of symptoms that people with all kinds of vasculitis may experience. These include:

• Fever that is unrelated to another illness
• Appetite reduction
• Unexplained weight loss
• Fatigue
• Generalized achiness

If vasculitis is focused in a particular organ or area of the body, symptoms can include:

• Skin: Bumps, red spots, itchiness, hives, bruising
• Joints: Pain or arthritis in the joints
• Lungs: Shortness of breath and coughing blood
• Abdomen: Ulcers and stomach pain
• Ear, nose and throat: Chronic ear infections, ulcers in the nose and hearing loss
• Eyes: Light sensitivity, blurry vision, redness, itch and burning
• Brain: Headaches, fuzzy thinking, altered mental ability, muscle weakness or paralysis
• Nerves: Numbness, tingling, weakness or loss of feeling in the extremities

Vasculitis is a condition that results when the body’s immune system turns on itself and mistakenly attacks its own blood vessels. It can be triggered by another infection, an autoimmune disease like lupus, or blood cancers like leukemia.

Risk Factors

Vasculitis can affect anyone at any time. It is found in people of all ages and in both sexes. Some forms of vasculitis are more common in people of different ages (like giant cell arteritis in individuals over the age of 50); gender (microscopic polyangiitis found more often in men than women); and ethnicity (Behcet’s disease, in people of Middle Eastern descent). Risk factors also include:

• Having medical conditions like hepatitis B or C
• Having other autoimmune diseases, like lupus and rheumatoid arthritis
• Smoking

Diagnostic Tests

There are a handful of tests used to determine if vasculitis is present. Some are general tests and others are more focused on various parts of the body that might be affected by the condition. These include:

• Blood tests: Used to determine if there are abnormal levels of protein in the blood, these include: hemoglobin and hematocrit (which show if there are low levels of red blood cells); antineutrophil cytoplasmic antibodies, present with certain types of vasculitis; and C-reactive protein and erythrocyte sedimentation rate, indicators that inflammation is present.
• Biopsy: In a biopsy, a physician takes a small sample of tissue from a blood vessel or organ. The tissue is then studied by a pathologist for inflammation or tissue damage.
• Urinalysis: This test checks for protein in the blood cells or urine to see if vasculitis might be present in the kidneys.
• Electrocardiogram, Echocardiogram and chest X-rays: These are tests that evaluate heart rhythm abnormalities, imaging of the heart as well as imaging of the lungs.
• Lung function tests: These tests are used to find out if air is moving freely in and out of your lungs or if there is blockage or problems with gas exchange between the blood vessels and the lungs.
• Ultrasound, CT scan and MRIs: These are imaging tests that create pictures of areas, such as the abdomen, to see if organs or blood vessels are affected.
• Angiograph: In this test, dye and X-rays are used to determine if blood is flowing properly through the blood vessels.

Complications

Complications of vasculitis vary depending upon where it is found in your body. Potential problems include kidney failure, blindness, aneurysm (bulge in the wall of the blood vessel), tissue damage and heart problems.

Some types of vasculitis are self-limited and can improve with minimal interventions such as bed rest or over the counter pain medication. Most require some sort of medical therapy.

Prescription medication

This is the most common medical treatment for vasculitis, and may include:

• Corticosteroids: Prednisone and prednisolone, may be used to reduce inflammation in the body.
• Cytotoxic medications: Other medications including azathioprine, methotrexate, rituximab, tocilizumab, and cyclophosphamide, among others, may be required to get the disease under long-term control.

Surgery

If vasculitis progresses to the point where blood vessels become too inflamed, the condition may result in an aneurysm (bulge in the vessel wall). If this occurs, surgery may be required to remove the aneurysm.

 

Anju Tripathi Peters, MD

Professor of Medicine (Allergy and Immunology) and Otolaryngology - Head and Neck Surgery

 

Anjum Koreishi, MD

Assistant Professor of Ophthalmology

Andrew Hoel, MD

Associate Professor of Surgery (Vascular Surgery)

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Assistant Professor of Otolaryngology 

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Click HERE to complete the CME video on EGPA (requires Medscape login)